Bilateral frontoparietal polymicrogyria, Lennox-Gastaut

(BFPP) has been reported in sporadic patients and in recessive pedigrees. Eleven mutations in GPR56, a gene encoding an evolutionarily dynamic G-pro-tein–coupled receptor, have been identified in 29 patients from 18 families. The clinical features of BFPP include severe mental retardation, motor and language impairment, and epilepsy. No detailed description of the epilepsy is available for the patients reported to date. We report three consanguineous families in which four affected individuals with BFPP and GPR56 mutations had Lennox-Gastaut syndrome. Methods: Family studies, brain magnetic reso-nance imaging (MRI), electroencephalography (EEG)-video recordings, and mutation analysis